Perioperative diagnosis and impact of acquired von Willebrand syndrome in infants with congenital heart disease.

Acquired von Willebrand syndrome (aVWS) has been reported in patients with congenital heart diseases associated with shear stress caused by significant blood flow gradients. Its etiology and impact on intraoperative bleeding during pediatric cardiac surgery have not been systematically studied. This single-center, prospective, observational study investigated appropriate diagnostic tools of aVWS compared with multimer analysis as diagnostic criterion standard and aimed to clarify the role of aVWS in intraoperative hemorrhage. A total of 65 newborns and infants aged 0 to 12 months scheduled for cardiac surgery at our tertiary referral center from March 2018 to July 2019 were included in the analysis. The glycoprotein Ib M assay (GPIbM)/von Willebrand factor antigen (VWF:Ag) ratio provided the best predictability of aVWS (area under the receiver operating characteristic curve [AUC], 0.81 [95% CI, 0.75-0.86]), followed by VWF collagen binding assay/VWF:Ag ratio (AUC, 0.70 [0.63-0.77]) and peak systolic echocardiographic gradients (AUC, 0.69 [0.62-0.76]). A cutoff value of 0.83 was proposed for the GPIbM/VWF:Ag ratio. Intraoperative high-molecular-weight multimer ratios were inversely correlated with cardiopulmonary bypass (CPB) time (r = -0.57) and aortic cross-clamp time (r = -0.54). Patients with intraoperative aVWS received significantly more fresh frozen plasma (P = .016) and fibrinogen concentrate (P = .011) than those without. The amounts of other administered blood components and chest closure times did not differ significantly. CPB appears to trigger aVWS in pediatric cardiac surgery. The GPIbM/VWF:Ag ratio is a reliable test that can be included in routine intraoperative laboratory workup. Our data provide the basis for further studies in larger patient cohorts to achieve definitive clarification of the effects of aVWS and its potential treatment on intraoperative bleeding.

The effect of intraoperative cerebral oxygen desaturations on postoperative cerebral oxygen metabolism in neonates and infants a pilot study.

INTRODUCTION: Cerebral oxygen desaturation during pediatric surgery has been associated with adverse perioperative outcomes. The aim of this pilot study was to analyze the frequency and severity of intraoperative cerebral oxygen desaturations and their impact on postoperative cerebral oxygen metabolism in neonates and infants undergoing pediatric surgery. METHODS: In a prospective pilot study, intra- and postoperative regional cerebral oxygen saturation and blood flow were measured noninvasively using a device combining laser Doppler flowmetry and white-light-spectrometry. Thirty-seven consecutive neonates and infants undergoing noncardiac surgery under general anesthesia for more than 30 min and necessity for invasive arterial blood pressure monitoring were included. Patients with pre-known congenital structural heart disease or cerebral disease were excluded. Continuously brain monitor recording was started in sedated patients before induction of anesthesia (preoperative baseline) and was completed 1 h postoperatively in the PICU in sedated, intubated, and mechanically ventilated states at the PICU (postoperative state). Baseline and postoperative state for cerebral fractional tissue oxygen extraction and approximated cerebral metabolic rate of oxygen were calculated. RESULTS: Seventeen (46%) of the 37 studied neonates and infants suffered from intraoperative periods of regional cerebral oxygen desaturation below 20% of the baseline (event group). Severity of cerebral desaturations was median 4.0%min/h [range 0.1-58.7; interquartile range [IQR] 0.99-21.29]. In the event group, the duration of surgery was significantly longer (median 135 min [range 11-260; IQR 113.5-167.0] vs median 46.5 min [range 11-180; IQR 30.5-159.3]; difference of -62.94; 95% confidence interval [CI] -105.17 to -20.71; p = .021). In the event group, cerebral fractional tissue oxygen extraction (median 0.41 [range 0.20-0.55; IQR 0.26-0.44] vs. median 0.27 [range 0.11-0.41; IQR 0.20-0.31]; difference of -0.11; 95% CI -0.17 to -0.05; p = .001) and approximated cerebral metabolic rate of oxygen (median 6.15 arbitrary unit [range 2.69-12.07; IQR 5.12-7.21] vs. median 4.14 arbitrary unit [range 1.78-7.86; IQR 3.82-6.31]; difference of -1.76; 95% CI -3.03 to -0.49; p = .009) were significantly higher and the cerebral regional oxygen saturation (median 58.99% [range 44.87-79.1; IQR 54.26-72.61] vs median 70.94% [range 57.9-86.13; IQR 67.07-76.59]; difference of 10.01; 95% CI 4.13-15.90; p = .002) significantly lower after surgery compared to the nonevent group. DISCUSSION: The increase of approximated cerebral metabolic rate of oxygen could indicate an elevated oxidative energy metabolism in the "stressed" brain, due to repair processes. The increased cerebral fractional tissue oxygen extraction fits with the decreased NIRS cerebral oxygenation. Our data suggest that an increase in cerebral oxygen metabolism was the cause. CONCLUSION: Cerebral oxygen desaturation during major surgery in neonates and infants is associated with early postoperative increased cerebral oxygen extraction and possibly increased cerebral oxygen metabolism.

Chronic Pleural Effusion in Ventriculoperitoneal Shunt due to Diaphragmatic CSF Fistula: Report of a Case Treated by Endoscopic Choroid Plexus Coagulation and Literature Review.

INTRODUCTION: Chronic pleural cerebrospinal fluid (CSF) effusion is a rare complication after ventriculoperitoneal (VP) shunt insertion and only 18 cases in children and adults have been described so far without catheter dislocation to the intrathoracic cavity. CASE PRESENTATION: We report on a 4-year-old girl with a complex history of underlying neurogenetic disorder, a hypoxic-ischemic encephalopathy after influenza A infection with septic shock and severe acute respiratory distress syndrome, followed by meningitis at the age of 10 months. In consequence, she developed a severe cerebral atrophy and post-meningitic hydrocephalus requiring placement of a VP shunt. At age 4, she was admitted with community-acquired mycoplasma pneumonia and developed increasing pleural effusions leading to severe respiratory distress and requiring continuous chest tube drainage (up to 1,000-1,400 mL/day) that could not be weaned. ß trace protein, in CSF present at concentrations >6 mg/L, was found in the pleural fluid at low concentrations of 2.7 mg/L. An abdomino-thoracic CSF fistula was finally proven by single photon emission computerized tomography combined with low-dose computer tomography. After shunt externalization, the pleural effusion stopped and the chest tube was removed. CSF production rate remains high above 500 mL/24 h. An atrial CSF shunt could not be placed, since a hemodynamically relevant atrial septum defect with frail circulatory balance would not have tolerated the large CSF volumes. Therefore, she underwent a total bilateral endoscopic choroid plexus laser coagulation (CPC) within the lateral ventricles via bi-occipital burr holes. Postoperatively CSF production rate went close to 0 mL and after external ventricular drain removal no signs and symptoms of hydrocephalus developed during a follow-up of now 2.5 years. CONCLUSION: In summary, pleural effusions in patients with VP shunt can rarely be caused by an abdomino-thoracic fistula, with non-elevated ß-trace protein in the pleural fluid. The majority of reported cases in literature were treated by ventriculoatrial shunt. This is the 2nd reported case, which has been successfully treated by radical CPC alone including the temporal horn choroid plexus, making the child shunt independent.

A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report.

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment. CASE PRESENTATION: We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months. CONCLUSIONS: Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.

Coronary Artery Anomalies and Their Impact on the Feasibility of Percutaneous Pulmonary Valve Implantation.

One of the major obstacles preventing successful percutaneous pulmonary valve implantation (PPVI) is related to the close proximity of coronary artery branches to the expected landing zone. The aim of this study was to assess the frequency of coronary artery anomalies (CAAs) especially those associated with major coronary branches crossing the right ventricular outflow tract (RVOT) and to describe their relevance for the feasibility of percutaneous pulmonary valve implantation (PPVI). In our retrospective single-center study 90 patients were evaluated who underwent invasive testing for PPVI in our institution from 1/2010 to 1/2020. CAAs were identified in seven patients (8%) associated with major branches crossing the RVOT due to origin of the left anterior descending (LAD) or a single coronary artery from the right aortic sinus. In 5/7 patients with CAAs balloon testing of the RVOT and selective coronary angiographies revealed a sufficiently large landing zone distal to the coronary artery branch. While unfavorable RVOT dimensions prevented PPVI in one, PPVI was performed successfully in the remaining four patients. The relatively short landing zone required application of the "folded" melody technique in two patients. All patients are doing well (mean follow-up 3 years). CAAs associated with major coronary branches crossing the RVOT can be expected in about 8% of patients who are potential candidates for PPVI. Since the LAD crossed the RVOT below the plane of the pulmonary valve successful distal implantation of the valve was possible in 4/7 patients. Therefore these coronary anomalies should not be considered as primary contraindications for PPVI.

Evaluation of a Multimodal Resuscitation Program and Comparison of Mouth-to-Mouth and Bag-Mask Ventilation by Relatives of Children With Chronic Diseases.

OBJECTIVES: Children with chronic critical illness are at higher risk for cardiopulmonary arrests. Before chronically ill children are discharged from hospital, family members receive training in basic life support at many institutions. We evaluated whether a multimodal training program is able to teach adherence to current resuscitation guidelines and whether laypersons can be trained to perform both bag-mask ventilation and mouth-to-mouth ventilation equally effective in infants. DESIGN: Prospective observational study. SETTING: Pediatric critical care unit of a tertiary referral center. SUBJECTS: Relatives of children with chronic illness prior to discharge from hospital. INTERVENTIONS: Multimodal emergency and cardiopulmonary resuscitation training program. MEASUREMENTS AND MAIN RESULTS: Following participation in our cardiopulmonary resuscitation training program 56 participants performed 112 simulated cardiopulmonary resuscitations (56 with mouth-to-mouth ventilation, 56 with bag-mask ventilation). Nearly all participants checked for consciousness and breathing. Shouting for help and activation of the emergency response system was only performed in half of the cases. There was almost full adherence to the resuscitation guidelines regarding number of chest compressions, chest compression rate, compression depth, full chest recoil, and duration of interruption of chest compression for rescue breaths. The comparison of mouth-to-mouth ventilation and bag-mask ventilation revealed no significant differences regarding the rate of successful ventilation (mouth-to-mouth ventilation: 77.1% ± 39.6%, bag-mask ventilation: 80.4% ± 38.0%; p = 0.39) and the cardiopulmonary resuscitation performance. CONCLUSIONS: A standardized multimodal cardiopulmonary resuscitation training program for family members of chronically ill children is effective to teach good cardiopulmonary resuscitation performance and adherence to resuscitation guidelines. Laypersons could be successfully trained to equally perform mouth-to-mouth and bag-mask ventilation technique.

Implementation of a Blood-Saving Program in a Pediatric Intensive Care Unit to Reduce Diagnostic Blood Loss.

BACKGROUND: We aimed to reduce blood loss in the pediatric critical care unit (PICU) due to blood sampling in neonates and infants. Therefore, an educational program for our staff was established and evaluated. METHODS: Patients in a PICU of a tertiary referral center aged 0-12 months who underwent surgery of congenital heart disease on cardiopulmonary bypass were enrolled and divided into a pre- and a post-implementation group. We assessed frequency and types of postoperative blood samples, required blood volume, and amount of blood transfusions in the PICU within 5 days after cardiac surgery. RESULTS: Populations were similar prior and after the implementation. Blood drawn for blood gas analysis (0,52 ml±0,16 vs. 0,38 ml±0,12, p<0,001) and for complete blood sampling (2,62 ml±0,32 vs. 2,11 ml±0,35, p<0,001) could be successfully reduced after implementation of our blood-saving program. The daily diagnostic blood loss per patient was significantly reduced by approximately 35% (1,7 ml/kg/d±1,0 vs. 1,1 ml/kg/d±0,7, p=0,008). DISCUSSION: Our quality improvement program is feasible and effective to significantly reduce the blood loss due to blood sampling. Although the incidence of red blood cell transfusions was not significantly reduced, it is certainly beneficial to try to reduce diagnostic blood loss, especially in children with complex diseases requiring long-term intensive care treatment. CONCLUSION: We could demonstrate that it is possible to significantly reduce the blood loss due to blood sampling with a simple educational program for PICU staff. HINTERGRUND: Unser Ziel war es, den Blutverlust durch Blutabnahmen auf der pädiatrischen Intensivstation bei Neugeborenen und Säuglingen zu reduzieren. Deshalb wurde ein Schulungsprogramm für unsere Mitarbeiter etabliert und ausgewertet. METHODE: Patienten unserer pädiatrischen Intensivstation im Alter von 0-12 Monaten nach einer Operation eines angeborenen Herzfehlers mit Herz-Lungen-Maschine, wurden eingeschlossen und in eine Gruppe vor und nach der Implementierung des Schulungsprogramms zugeteilt. Wir haben die Häufigkeit und Art der postoperativen Blutproben, das benötigte Blutvolumen und die Menge der Bluttransfusionen auf der Intensivstation innerhalb von 5 Tagen nach der Herzoperation ausgewertet. ERGEBNISSE: Die Patientencharakteristik beider Gruppen zeigte keine relevanten Unterschiede. Blut, das für Blutgasanalysen (0,52 ml±0,16 vs. 0,38 ml±0,12, p<0,001) und für vollständige Blutentnahmen (2,62 ml±0,32 vs. 2,11 ml±0,35, p<0,001) entnommen wurde, konnte nach Umsetzung unseres Blutsparprogramms erfolgreich reduziert werden. Der tägliche diagnostische Blutverlust pro Patienten wurde signifikant um ca. 35% reduziert (1,7 ml/kg/d±1,0 vs. 1,1 ml/kg/d±0,7, p=0,008). DISKUSSION: Unser Schulungsprogramm für Mitarbeiter ist einfach umzusetzen und effektiv, den Blutverlust durch Blutentnahmen deutlich zu reduzieren. Obwohl die Inzidenz von Bluttransfusionen nicht signifikant reduziert wurde, ist es sicherlich erstrebenswert, den diagnostischen Blutverlust insbesondere bei Kindern mit komplexen Krankheiten, die eine langfristige Intensivbehandlung erfordern, zu reduzieren. SCHLUSSFOLGERUNG: Wir konnten zeigen, dass es möglich ist, den Blutverlust durch Blutentnahme mit einem einfachen Schulungsprogramm für Mitarbeiter auf der Intensivstation deutlich zu reduzieren.

Perioperative Assessment of Cerebral Oxygen Metabolism in Infants With Functionally Univentricular Hearts Undergoing the Bidirectional Cavopulmonary Connection.

OBJECTIVES: The transition from single-ventricle lesions with surgically placed systemic-to-pulmonary artery shunt to the circulation following a bidirectional cavopulmonary connection results in higher pressure in the superior vena cava when compared with the preceding circulation. The aim of this study was to evaluate the impact of this transition on the perioperative cerebral oxygen metabolism. DESIGN: Prospective observational cohort study. SETTING: Pediatric critical care unit of a tertiary referral center. PATIENTS: Sixteen infants after bidirectional cavopulmonary connection. INTERVENTION: Cardiac surgery (bidirectional cavopulmonary connection). MEASUREMENTS AND MAIN RESULTS: We measured regional cerebral oxygen saturation, amount of hemoglobin, blood flow velocity, and microperfusion immediately before, 12-24 hours, and 36-48 hours following bidirectional cavopulmonary connection. Based on these measurements, we calculated cerebral fractional tissue oxygen extraction and approximated cerebral metabolic rate of oxygen. Mean pressure in the superior vena cava increased significantly (8 vs 17 mm Hg; p < 0.001) following bidirectional cavopulmonary connection. Mean cerebral oxygen saturation increased from 49.0% (27.4-61.0) to 56.9% (39.5-64.0) (p = 0.008), whereas mean cerebral blood flow velocity decreased from 80.0 arbitrary units (61.9-93.0) to 67.3 arbitrary units (59.0-83.3) (p < 0.001). No change was found in the cerebral amount of hemoglobin and in the cerebral microperfusion. Mean cerebral fractional tissue oxygen extraction (0.48 [0.17-0.63] vs 0.30 [0.19-0.56]; p = 0.006) and approximated cerebral metabolic rate of oxygen (5.82 arbitrary units [2.70-8.78] vs 2.27 arbitrary units [1.19-7.35]; p < 0.001) decreased significantly. CONCLUSIONS: Establishment of bidirectional cavopulmonary connection is associated with postoperative improvement in cerebral oxygen metabolism. Cerebral amount of hemoglobin did not increase, although creation of the bidirectional cavopulmonary connection results in significant elevation in superior vena cava pressure. Improvement in cerebral oxygen metabolism was due to lower cerebral blood flow velocity and stable microperfusion, which may indicate intact cerebral autoregulation.

[Medical Staffing in Neonatal and Pediatric Intensive Care Units - an European Survey]. / Personalsituation auf neonatologischen und pädiatrischen Intensivstationen ­ eine europaweite Umfrage.

BACKGROUND: Recently, there is a growing scarcity of qualified personnel in German healthcare system. This can lead to problems especially in the highly specialized neonatal and pediatric intensive care. METHODS: We conducted a survey to evaluate the staffing situation at 583 European neonatal and pediatric intensive care units (ICU). RESULTS: Response rate was 48.5%. As a main result our evaluation demonstrated a significant lower nurse-staffing in Germany compared to the other countries (90.4 vs. 95.8%, p<0.001). In addition, the average nurse-to-patient ratio was worse in German neonatal ICUs (3.0 vs. 2.3, p<0.001). The presence of senior doctors is also lower in German neonatal and pediatric ICUs compared to the other countries (on weekdays: 12.0 vs. 14.6 h, p=0.04; on weekends: 8.9 vs. 13.2 h, p=0.003). DISCUSSION: Our survey demonstrates that the nurse-staffing at neonatal and ICUs in Germany is worse than in other European countries. The resulting potential loss of quality is worsened due to lower presence of senior doctors at the ICUs. Possible consequences include increase of complications, impairment of treatment success, closure of intensive care beds, and mental and physical illness of the staff. CONCLUSION: Compared to other European countries, there is a lack of nurses at neonatal and pediatric ICUs in Germany. This study underlines the importance to take measures to counteract nursing crisis.

Cerebral Oxygen Metabolism Before and After RBC Transfusion in Infants Following Major Surgical Procedures.

OBJECTIVE: Although infants following major surgery frequently require RBC transfusions, there is still controversy concerning the best definition for requirement of transfusion in the individual patient. The aim of this study was to determine the impact of RBC transfusion on cerebral oxygen metabolism in noncardiac and cardiac postsurgical infants. DESIGN: Prospective observational cohort study. SETTING: Pediatric critical care unit of a tertiary referral center. PATIENTS: Fifty-eight infants (15 after pediatric surgery and 43 after cardiac surgery) with anemia requiring RBC transfusion were included. INTERVENTIONS: RBC transfusion. MEASUREMENTS AND MAIN RESULTS: We measured noninvasively regional cerebral oxygen saturation and microperfusion (relative cerebral blood flow) using tissue spectrometry and laser Doppler flowmetry before and after RBC transfusion. Cerebral fractional tissue oxygen extraction and approximated cerebral metabolic rate of oxygen were calculated. Fifty-eight RBC transfusions in 58 patients were monitored (15 after general surgery, 24 after cardiac surgery resulting in acyanotic biventricular physiology and 19 in functionally univentricular hearts including hypoplastic left heart following neonatal palliation). The posttransfusion hemoglobin concentrations increased significantly (9.7 g/dL vs 12.8 g/dL; 9.7 g/dL vs 13.8 g/dL; 13.1 g/dL vs 15.6 g/dL; p < 0.001, respectively). Posttransfusion cerebral oxygen saturation was significantly higher than pretransfusion (61% [51-78] vs 72% [59-89]; p < 0.001; 58% [35-77] vs 71% [57-88]; p < 0.001; 51% [37-61] vs 58% [42-73]; p = 0.007). Cerebral fractional tissue oxygen extraction decreased posttransfusion significantly 0.37 (0.16-0.47) and 0.27 (0.07-039), p = 0.002; 0.40 (0.2-0.62) vs 0.26 (0.11-0.57), p = 0.001; 0.42 (0.23-0.52) vs 0.32 (0.1-0.42), p = 0.017. Cerebral blood flow and approximated cerebral metabolic rate of oxygen showed no significant change during the observation period. The increase in cerebral oxygen saturation and the decrease in cerebral fractional tissue oxygen extraction were most pronounced in patients after cardiac surgery with a pretransfusion cerebral fractional tissue oxygen extraction greater than or equal to 0.4. CONCLUSION: Following RBC transfusion, cerebral oxygen saturation increases and cerebral fractional tissue oxygen extraction decreases. The data suggest that cerebral oxygenation in postoperative infants with cerebral fractional tissue oxygen extraction greater than or equal to 0.4 may be at risk in instable hemodynamic or respiratory situations.

Perioperative Cerebral Oxygenation Metabolism in Neonates with Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries.

Optimizing oxygen delivery to the brain is one of the main goals in children with congenital heart defects after surgery. It has been shown that cerebral oxygen saturation (cSO2) is depressed within the first day after neonatal cardiopulmonary bypass surgery. However, peri-operative cerebral oxygen metabolism has not yet been assessed in previous studies. The aim of this study was to describe the peri-operative changes in cerebral oxygen metabolism in neonates with congenital heart defects following cardiopulmonary bypass surgery. Prospective observational cohort study. PICU of a tertiary referral center. Fourteen neonates with hypoplastic left heart syndrome (HLHS) undergoing Norwood procedure and 14 neonates with transposition of great arteries (TGA) undergoing arterial switch operation (ASO) were enrolled. Pediatric heart surgery. We measured non-invasively regional cSO2 and microperfusion (rcFlow) using tissue spectrometry and laser Doppler flowmetry before and after surgery. Cerebral fractional tissue oxygen extraction (cFTOE), the arterio-cerebral difference in oxygen content (acDO2) and approximated cerebral metabolic rate of oxygen (aCMRO2) were calculated. According to the postsurgical hemodynamics, arterial saturation (aSO2) normalized immediately after surgery in the TGA group, whereas HLHS patients still were cyanotic. cSO2 significantly increased in TGA group over 48 h after ASO (p = 0.004) and was significantly higher compared to HLHS group after Norwood procedure. cFTOE as a risk marker for brain injury was elevated before surgery (TGA group 0.37 ± 0.10, HLHS group 0.42 ± 0.12) and showed a slight decrease after ASO (p = 0.35) but significantly decreased in patients after Norwood procedure (p = 0.02). Preo-peratively, acDO2 was significantly higher in patients with HLHS compared to patients with TGA (7.7 ± 2.5 vs. 5.2 ± 1.6 ml/dl, p = 0.005), but normalized in the posto-perative course. Before surgery, the aCMRO2 was slightly higher in the HLHS group (5.1 ± 1.5 vs. 3.9 ± 2.5 AU, p = 0.14), but significantly decreased after Norwood procedure (- 1.6 AU, p = 0.009). There was no difference in rcFlow between both groups and between the points in time prior and after surgery. Neonates undergoing cardiac surgery suffer from peri-operative changes in hemodynamics and cerebral hypoxemic stress. The cerebral oxygen metabolism seems to be more affected in cyanotic children with functionally univentricular hearts compared to post-operative acyanotic patients. Additional stress factors must be avoided to achieve the best possible neurological outcome.

Severe Upper Airway Obstruction After Intraoperative Transesophageal Echocardiography in Pediatric Cardiac Surgery: A Retrospective Analysis.

OBJECTIVES: The aim of this study was to evaluate if there is a correlation between the use of intraoperative transesophageal echocardiography and an increased rate of extubation failure and to find other risk factors for severe upper airway obstructions after pediatric cardiac surgery. DESIGN: Retrospective analysis. SETTING: Cardiac PICU. PATIENTS: Patients 24 months old or younger who underwent surgery for congenital heart disease with cardiopulmonary bypass were retrospectively enrolled and divided into two groups depending on whether they received an intraoperative transesophageal echocardiography or not. We analyzed all cases of early reintubations within 12 hours after extubation due to a documented upper airway obstruction. INTERVENTION: None. MEASUREMENTS AND MAIN RESULTS: From a total of 424 patients, 12 patients (2.8%) met our criteria of early reintubation due to upper airway obstruction. Ten of 207 children in the transesophageal echocardiography group had to be reintubated, whereas only two of the 217 children in the control group had to be reintubated (4.8% vs 0.9%; p = 0.018). Logistic regression analysis showed a significant correlation between use of intraoperative transesophageal echocardiography and extubation failure (odds ratio, 5.64; 95% CI, 1.18-27.05; p = 0.030). There was no significant relationship among sex (odds ratio, 4.53; 95% CI, 0.93-22.05; p = 0.061), weight (odds ratio, 1.07; 95% CI, 0.82-1.40; p = 0.601), duration of surgery (odds ratio, 1.04; 95% CI, 0.74-1.44; p = 0.834), duration of mechanical ventilation (odds ratio, 1.00; 95% CI, 0.99-1.00; p = 0.998), and occurrence of trisomy 21 (odds ratio, 3.47; 95% CI, 0.83-14.56; p = 0.089). CONCLUSIONS: Although the benefits of intraoperative transesophageal echocardiography during pediatric cardiac surgery are undisputed, it may be one factor which could increase the rate of severe upper airway obstruction after extubation with the need for reintubation. We suggest to take precautions before extubating high-risk patients, especially in young male children with genetic abnormalities after cardiac surgery with cardiopulmonary bypass.

Nurse-driven analgesia and sedation in pediatric patients with univentricular hearts requiring extracorporeal life support after first-stage palliation surgery: A pilot study.

BACKGROUND: Few data are available regarding requirements of sedation and analgesia in children during extracorporeal life support. AIMS: The aim of this study was to evaluate if children with functionally univentricular hearts on extracorporeal life support after first-stage palliation surgery have higher requirement of analgesics and sedatives compared with children without extracorporeal life support using a goal-directed nurse-driven analgesia and sedation protocol. METHODS: This prospective observational matched case-control pilot study was conducted at a cardiac pediatric intensive care unit of a tertiary referral center. Seventeen patients with functionally univentricular hearts including hypoplastic left heart syndrome who were on extracorporeal life support after first-stage palliation surgery were enrolled from July 2012 to January 2017. Seventeen matched patients served as controls. Doses of morphine, midazolam, clonidine, and muscle relaxants as well as sedation scores (COMFORT behavior scale and the nurse interpretation of sedation scale) were assessed according to a nurse-driven protocol every 8 hours up to 120 hours after first-stage palliation surgery. RESULTS: Sedation scores were equal in the extracorporeal life support group and in the control group at most points in time. There was no significant difference in cumulative doses of morphine and midazolam. However, children of the extracorporeal life support group received higher doses of midazolam and morphine at some points in time. CONCLUSION: A nurse-driven protocol for analgesia and sedation of children with extracorporeal life support is feasible. Patients with extracorporeal life support do not need deeper sedation levels and have not higher cumulative sedation requirements than children without extracorporeal life support.

Vascular rehabilitation in children with chronic intestinal failure reduces the risk of central-line associated bloodstream infections and catheter replacements.

BACKGROUND & AIMS: Children with chronic intestinal failure (IF) require a long-term central venous catheter (CVC) for provision of parenteral nutrition. Vascular, mechanical and infectious complications such as central line-associated bloodstream infections (CLABSI) may lead to progressive loss of venous access sites. Handling and management of CVCs therefore play an important role. Our vascular rehabilitation concept (VRC) is a core component of our intestinal rehabilitation program (IRP) and consists of an education program, optimization of skin care, catheter fixation and lock solution, and the use of hybrid technique for catheter placement. Aim of this study is to analyse the effectiveness of our VRC on CLABSI rates and need for CVC replacements. METHODS: Retrospective analysis of all children treated in our IRP that were followed up between 2018 and 2023. RESULTS: A total of 117 children with chronic IF could be included for analysis of 248864 catheter days (CD). 91 patients were referred from other hospitals (127117 CD before and 89359 CD after entry into our IRP). Children receiving primary care at our IRP (32388 CD) showed a significantly lower CLABSI and line replacement rate than patients referred from external centers (p < 0.001). After entering our IRP, CLABSI rates and need for CVC replacements per 1000 CD decreased significantly in referred patients: 1.19 to 0.26 and 1.77 to 0.59, respectively (p < 0.001). CONCLUSION: Management of paediatric chronic IF patients by an IRP with a vascular rehabilitation concept significantly lowers the rate of CLABSI episodes and the need for catheter replacements.

Successful on-ECLS Repair of CDH and Omphalocele in a Newborn.

Both congenital diaphragmatic hernias (CDHs) and omphaloceles show relevant overall mortality rates as individual findings. The combination of the two has been described only sparsely in the literature and almost always with a fatal course. Here, we describe a term neonate with a rare high-risk constellation of left-sided CDH and a large omphalocele who was successfully treated on extracorporeal life support (ECLS). Prenatally, the patient was diagnosed with a large omphalocele and a left CDH with a lung volume of ∼27% and an observed to expected lung-to-head ratio of 30%. Due to respiratory insufficiency, an ECLS device was implanted. As weaning from ECLS was not foreseeable, the female infant underwent successful surgery on ECLS on the ninth day of life. Perioperative high-frequency oscillatory ventilation and circulatory and coagulation management under point-of-care monitoring were the main anesthesiological challenges. Over the following 3 days, ECLS weaning was successful, and the patient was extubated after another 43 days. Surgical treatment on ECLS can expand the spectrum of therapy in high-risk constellations if potential risks are minimized and there is close interdisciplinary cooperation.

Thoracic lymphatic anomalies in patients with univentricular hearts: correlation of morphologic findings in isotropic T2-weighted MRI with the outcome after fontan palliation.

Objectives: In this study we examined the correlation between the extent of thoracic lymphatic anomalies in patients after surgical palliation by total cavopulmonary connection (TCPC) and their outcome in terms of clinical and laboratory parameters. Materials and methods: We prospectively examined 33 patients after TCPC with an isotropic heavily T2-weighted MRI sequence on a 3.0 T scanner. Examinations were performed after a solid meal, slice thickness of 0.6 mm, TR of 2400 ms, TE of 692 ms, FoV of 460 mm, covering thoracic and abdominal regions. Findings of the lymphatic system were correlated with clinical and laboratory parameters obtained at the annual routine check-up. Results: Eight patients (group 1) showed type 4 lymphatic abnormalities. Twentyfive patients (group 2) presented less severe anomalies (type 1-3). In the treadmill CPET, group 2 reached step 7.0;6.0/8.0 vs. 6.0;3.5/6.8 in group 1 (p = 0.006*) and a distance of 775;638/854 m vs. 513;315/661 m (p = 0.006*). In the laboratory examinations, group 2 showed significantly lower levels of AST, ALT and stool calprotectin as compared to group 1. There were no significant differences in NT-pro-BNP, total protein, IgG, lymphocytes or platelets, but trends. A history of ascites showed 5/8 patients in group 1 vs. 4/25 patients in group 2 (p = 0.02*), PLE occurred in 4/8 patient in group 1 vs. 1/25 patients in group 2 (p = 0.008*). Conclusion: In the long-term follow-up after TCPC, patients with severe thoracic and cervical lymphatic abnormalities showed restrictions in exercise capacity, higher liver enzymes and an increased rate of symptoms of imminent Fontan-failure such as ascites and PLE.

Current Knowledge Regarding Long-Term Consequences of Pediatric Intensive Care: A Staff Survey in Intensive Care Units in German-Speaking Countries.

Background: The Post Intensive Care Syndrome (PICS) describes new impairments of physical, cognitive, social, or mental health after critical illness. In recent years, prevention and therapy concepts have been developed. However, it is unclear whether and to what extent these concepts are known and implemented in hospitals in German-speaking countries. Methods: We conducted an anonymous online survey in German-speaking pediatric intensive care units on the current state of knowledge about the long-term consequences of intensive care treatment as well as about already established prevention and therapy measures. The request to participate in the survey was sent to the heads of the PICUs of 98 hospitals. Results: We received 98 responses, 54% of the responses came from nurses, 43% from physicians and 3% from psychologist, all working in intensive care. As a main finding, our survey showed that for only 31% of the respondents PICS has an importance in their daily clinical practice. On average, respondents estimated that about 42% of children receiving intensive care were affected by long-term consequences after intensive care. The existence of a follow-up outpatient clinic was mentioned by 14% of the respondents. Frequent reported barriers to providing follow-up clinics were lack of time and staff. Most frequent mentioned core outcome parameters were normal developmental trajectory (59%) and good quality of life (52%). Conclusion: Overall, the concept of PICS seems to be underrepresented in German-speaking pediatric intensive care units. It is crucial to expand knowledge on long-term complications after pediatric critical care and to strive for further research through follow-up programs and therewith ultimately improve long-term outcomes.

Implementation and Evaluation of Resuscitation Training for Childcare Workers.

Background and Objective: Children spend a large amount of time in daycare centers or schools. Therefore, it makes sense to train caregivers well in first-aid measures in children. The aim of this study is to evaluate whether a multimodal resuscitation training for childcare workers can teach adherence to resuscitation guidelines in a sustainable way. Materials and Methods: Caregivers at a daycare center who had previously completed a first-aid course received a newly developed multimodal resuscitation training in small groups of 7-8 participants by 3 AHA certified PALS instructors and providers. The 4-h focused retraining consisted of a theoretical component, expert modeling, resuscitation exercises on pediatric manikins (Laerdal Resusci Baby QCPR), and simulated emergency scenarios. Adherence to resuscitation guidelines was compared before retraining, immediately after training, and after 6 months. This included evaluation of chest compressions per round, chest compression rate, compression depth, full chest recoil, no-flow time, and success of rescue breaths. For better comparability and interpretation of the results, the parameters were evaluated both separately and summarized in a resuscitation score reflecting the overall adherence to the guidelines. Results: A total of 101 simulated cardiopulmonary resuscitations were evaluated in 39 participants. In comparison to pre-retraining, chest compressions per round (15.0 [10.0-29.0] vs. 30.0 [30.0-30.0], p < 0.001), chest compression rate (100.0 [75.0-120.0] vs. 112.5 [105-120.0], p < 0.001), correct compression depth (6.7% [0.0-100.0] vs. 100.0% [100.0-100.0], p < 0.001), no-flow time (7.0 s. [5.0-9.0] vs. 4.0 s. [3.0-5.0], p < 0.001), success of rescue breaths (0.0% [0.0-0.0] vs. 100.0% [100.0-100.0], p < 0.001), and resuscitation score were significantly improved immediately after training (3.9 [3.2-4.9] vs. 6.3 [5.6-6.7], p < 0.001). At follow-up, there was no significant change in chest compression rate and success of rescue breaths. Chest compressions per round (30.0 [15.0-30.0], p < 0.001), no-flow time (5.0 s. [4.0-8.0], p < 0.001), compression depths (100.0% [96.7-100.0], p < 0.001), and resuscitation score worsened again after 6 months (5.7 [4.7-6.4], p = 0.03). However, the results were still significantly better compared to pre-retraining. Conclusion: Our multimodal cardiopulmonary resuscitation training program for caregivers is effective to increase the resuscitation performance immediately after training. Although the effect diminishes after 6 months, adherence to resuscitation guidelines was significantly better than before retraining.

Modified ABCDEF-Bundles for Critically Ill Pediatric Patients - What Could They Look Like?

Background and Significance: Advances in pediatric intensive care have led to markedly improved survival rates in critically ill children. Approximately 70% of those children survive with varying forms of complex chronic diseases or impairment/disabilities. Length of stay, length of mechanical ventilation and number of interventions per patient are increasing with rising complexity of underlying diseases, leading to increasing pain, agitation, withdrawal symptoms, delirium, immobility, and sleep disruption. The ICU-Liberation Collaborative of the Society of Critical Care Medicine has developed a number of preventative measures for prevention, early detection, or treatment of physical and psychiatric/psychological sequelae of oftentimes traumatic intensive care medicine. These so called ABCDEF-Bundles consist of elements for (A) assessment, prevention and management of pain, (B) spontaneous awakening and breathing trials (SAT/SBT), (C) choice of analgesia and sedation, (D) assessment, prevention and management of delirium, (E) early mobility and exercise and (F) family engagement and empowerment. For adult patients in critical care medicine, research shows significant effects of bundle-implementation on survival, mechanical ventilation, coma, delirium and post-ICU discharge disposition. Research regarding PICS in children and possible preventative or therapeutic intervention is insufficient as yet. This narrative review provides available information for modification and further research on the ABCDEF-Bundles for use in critically ill children. Material and Methods: A narrative review of existing literature was used. Results: One obvious distinction to adult patients is the wide range of different developmental stages of children and the even closer relationship between patient and family. Evidence for pediatric ABCDEF-Bundles is insufficient and input can only be collected from literature regarding different subsections and topics. Conclusion: In addition to efforts to improve analgesia, sedation and weaning protocols with the aim of prevention, early detection and effective treatment of withdrawal symptoms or delirium, efforts are focused on adjusting ABCDEF bundle for the entire pediatric age group and on strengthening families' decision-making power, understanding parents as a resource for their child and involving them early in the care of their children.

Implementation of a Delirium Bundle for Pediatric Intensive Care Patients.

BACKGROUND AND OBJECTIVE: Delirium represents the most common form of acute cerebral dysfunction in critical illness. The prevention, recognition, and treatment of delirium must become the focus of modern pediatric intensive care, as delirium can lead to increased morbidity and mortality. The aim of this study is to evaluate the impact of a delirium bundle consisting of mainly non-pharmacological measures. MATERIAL AND METHODS: This is a pre-/post-implementation study in an interdisciplinary pediatric intensive care unit of a tertiary care university hospital. In the pre-implementation period, pediatric intensive care delirium was monitored using the Sophia Observation withdrawal Symptoms and Pediatric Delirium scale. After introduction of a delirium bundle consisting of non-pharmacological prevention and treatment measures a period of 4 months was interposed to train the PICU staff and ensure that the delirium bundle was implemented consistently before evaluating the effects in the post-implementation period. Data collection included prevalence of delirium and withdrawal, length of PICU stay, duration of mechanical ventilation, and cumulative dose of sedatives and analgesics. RESULTS: A total of 792 critically ill children aged 0-18 years were included in this study. An overall delirium prevalence of 30% was recorded in the pre-implementation group and 26% in the post-implementation group (p = 0.13). A significant reduction in the prevalence of pediatric delirium from was achieved in the subgroup of patients under 5 years of age (27.9 vs. 35.8%, p = 0.04) and in patients after surgery for congenital heart disease (28.2 vs. 39.5%, p = 0.04). Young age, length of PICU stay, and iatrogenic withdrawal syndrome were found to be risk factors for developing delirium. CONCLUSIONS: Based on a validated delirium monitoring, our study gives new information regarding the prevalence of pediatric delirium and the characteristics of intensive care patients at risk for this significant complication. Especially young patients and patients after surgery for congenital heart disease seem to benefit from the implementation of non-pharmacological delirium bundles. Based on our findings, it is important to promote change in pediatric intensive care-toward a comprehensive approach to prevent delirium in critically ill children as best as possible.